Fulminant hepatic failure

Incidence

Age

Sex

Geography

Aetiology - severe hepatic failure in which encephalopathy develops in under two weeks in a patient with a previously healthy liver
- due to acute hepatitis of any cause - most commonly viral hepatitis or paracetamol overdose
- rare in HCV

Presentation Examination
- jaundiced patient with a small liver and signs of hepatic encephalopathy
- mental state varies: drowsiness and confusion - unresponsive coma and convulsions
- fever, vomiting, hypotension, hypoglycaemia
- foetor hepaticus is common
- ascites and splenomegaly are rare
- spasticity of arms and legs; plantars remain flexor until late

Investigations
LFTs
- hyperbilirubinaemia
- high AST/ALT initially; however, these fall off with progression of the liver damage

Clotting screen
- low levels of factors -> high PTT

Serum albumin
- decreases with increasing liver damage

EEG
- may be useful in evaluating encephalopathy

USS
- liver size
- any underlying pathology

Macro

Micro - multiacinar necrosis affecting a large proportion of the liver

Staging

Serum markers

Management
Supportive
- should be undertaken in a specialised unit
- measure ICP directly; if signs of raised ICP, 20% mannitol 1 g/kg should be infused
- anticipate hypoglycaemia, hypokalaemia and hypocalcaemia; correct with 10% dextrose infusion, potassium and calcium
- coagulopathy managed by iv vitamin K, platelets, blood or FFP
- H2 antagonists are given to reduce GI bleeding
- treat infection with appropriate antibiotics
- treat renal and respiratory failure
- flumazenil can give a temporary imporvement in encephalopathy

Surgical
- liver transplant
- timing/necessity

Prognosis
Grades I and II encephalopathy
- 2/3 of patients survive

Grades III and IV encephalopathy
- related to aetiology
- generally survival is 70% in paracetamol overdose, 30-40% in HAV/HBV hepatitis

Poor prognostic indicators
- non A/E viral hepatitis / drug reaction
- age < 10 years or > 40 years
- short time interval from onset of jaundice to encephalopathy
- serum bilirubin > 300 µmol/L
- PT > 50s

Poor prognostic indicators in paracetamol OD -> consider for transplantation
- arterial pH < 7.3
- serum creatinine > 300 µmol/L
- PT > 100s
- grade III/IV encephalopathy

Complications - 80% of patients have cerebral oedema -> brain herniation -> death
- bacterial infections
- GI bleeing
- respiratory arrest
- renal failure - hepatorenal syndrome with ATN
- pancreatitis

Hepatobiliary medicine

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Incidence
Age
Sex
Geography
Aetiology	- severe hepatic failure in which encephalopathy develops in under two weeks in a patient with a previously healthy liver - due to acute hepatitis of any cause - most commonly viral hepatitis or paracetamol overdose - rare in HCV
Presentation	Examination - jaundiced patient with a small liver and signs of hepatic encephalopathy - mental state varies: drowsiness and confusion - unresponsive coma and convulsions - fever, vomiting, hypotension, hypoglycaemia - foetor hepaticus is common - ascites and splenomegaly are rare - spasticity of arms and legs; plantars remain flexor until late
Investigations	LFTs - hyperbilirubinaemia - high AST/ALT initially; however, these fall off with progression of the liver damage Clotting screen - low levels of factors -> high PTT Serum albumin - decreases with increasing liver damage EEG - may be useful in evaluating encephalopathy USS - liver size - any underlying pathology
Macro
Micro	- multiacinar necrosis affecting a large proportion of the liver
Staging
Serum markers
Management	Supportive - should be undertaken in a specialised unit - measure ICP directly; if signs of raised ICP, 20% mannitol 1 g/kg should be infused - anticipate hypoglycaemia, hypokalaemia and hypocalcaemia; correct with 10% dextrose infusion, potassium and calcium - coagulopathy managed by iv vitamin K, platelets, blood or FFP - H2 antagonists are given to reduce GI bleeding - treat infection with appropriate antibiotics - treat renal and respiratory failure - flumazenil can give a temporary imporvement in encephalopathy Surgical - liver transplant - timing/necessity
Prognosis	Grades I and II encephalopathy - 2/3 of patients survive Grades III and IV encephalopathy - related to aetiology - generally survival is 70% in paracetamol overdose, 30-40% in HAV/HBV hepatitis Poor prognostic indicators - non A/E viral hepatitis / drug reaction - age < 10 years or > 40 years - short time interval from onset of jaundice to encephalopathy - serum bilirubin > 300 µmol/L - PT > 50s Poor prognostic indicators in paracetamol OD -> consider for transplantation - arterial pH < 7.3 - serum creatinine > 300 µmol/L - PT > 100s - grade III/IV encephalopathy
Complications	- 80% of patients have cerebral oedema -> brain herniation -> death - bacterial infections - GI bleeing - respiratory arrest - renal failure - hepatorenal syndrome with ATN - pancreatitis